This is also known as Linear IgA bullous dermatosis (LABD). It present
with two variants. The childhood variant is known as chronic bullous dermatosis
of childhood or juvenile dermatitis herpetiformis. It arises within
the first decade of life with large, tense bullae on an erythematous base
and cocurs on the perioral, genital skin, lower abdomen and thighs. There
is a characteristic grouping called a cluster of jewels with polycyclic
grouping. Although benign, the lesions may persist for several months or years
and may rarely persist to puberty. If there is severe scarring, the term childhood
cicatricial pemphigoid has been used. There may be an association with
HLA antigen B8.

The adult variant is characterized by lesions resembling dermatitis herpetiformis
and bullous pemphigoid. These usually annular lesions involve the trunk, limbs,
oral mucosa, and conjunctiva. Facial and perineal involvement are uncommon.
Pruritis and burning may be present. This is a chronic disorder and lesions
may persist indefinitely. There is a lesser association with HLA B8.

Both variants arise from the binding of IgA to two dermal antigens. The first
is a LAD-1, a 97 kd protein within the anchoring filament protein ladinin.
The second is a 290 kd antigen in type VII collagen. These circulating autoantibodies
are found in 70% of childhood cases and 20% of adult cases. Unlike dermatitis
herpetiformis, there are no circulating antiendomysial antibodies nor is there
a gluten sensitive enteropathy. There is an association with other autoimmune
disorders and various drugs which all cause flares usually occurring within
4-14 days following ingestion. This drug associated variant may be the most
common form of the disease.

This is a subepidermal blistering disease with histologic features indistinguishable
from dermatitis herpetiformis.
Thus direct immunofluorescence (DIF) is needed to confirm the diagnosis. Dermal
papillary microabscesses are present leading the subepidermal cleft and blister
formation. DIF reveals a linear staining for IgA at the dermoepidermal junction.
In 20% of cases, there is also IgG, IgM, and C3.

As mentioned, distinction from dermatitis herpetiformis is necessary. IgA
is also deposited along the basement of eccrine secretory coils in patients
with alcoholic liver disease, a potential false positive.

BACKGROUND:
Linear IgA bullous dermatosis (LAD) of childhood is a rare acquired
subepidermal blistering disease of young children. Most of the studies
were reported from the USA and European countries.

METHOD: Twelve cases of Thai patients diagnosed as LAD of childhood
were analyzed concerning clinical, histopathological, immunopathological
findings including treatment responses and courses compared with those
of Caucasians.

RESULT: The mean age of onset was 5.1 years. The areas of common involvement
were the perioral region, lower abdomen, perineum, buttock, inner thighs
and extremities. Histopathology in half of the cases showed features
of dermatitis herpetiformis or bullous pemphigoid. All patients had
positive linear IgA band at the basement membrane zone (BMZ) by direct
immunofluorescence. Only one patient had positive circulating anti BMZ
antibody at the titer of 1:10. Most patients responded well to dapsone.
The mean duration before remission was 1.9 years.

CONCLUSION: Our study in Thai patients with LAD of childhood produced
data similar to previous studies carried out in the Caucasian nations.

Linear IgA disease
(LAD) is a well recognized subepidermal blistering disorder characterized
by linear deposits of IgA at the basement membrane zone. The aetiology
is unknown but there is a recognized association with lymphoproliferative
malignancies.

We report a case of LAD occurring in a patient with multicentric Castleman's
disease (angiofollicular lymph node hyperplasia), an association not
previously recorded in the literature.

BACKGROUND: Linear IgA bullous dermatosis is an autoimmune blistering disease characterized clinically by the presence of small tense blisters and immunologically by the presence of IgA at the dermal-epidermal junction. Idiopathic, systemic disease-related, and drug-related versions of this disorder have been described, with the latter most commonly associated with vancomycin.

OBSERVATIONS: We describe 2 patients with vancomycin-associated linear IgA bullous dermatosis who presented with a morbilliform eruption that lacked blistering. Lesional and perilesional tissue from each patient was examined by light microscopy and direct immunofluorescence. Histopathologic examination findings revealed vacuolar interface dermatitis with a mixed inflammatory infiltrate and occasional eosinophils, consistent with a drug eruption. Direct immunofluorescence revealed IgA deposited in a linear pattern at the dermoepidermal junction. In both patients, the results of indirect immunofluorescence using both IgG and IgA were negative.

CONCLUSIONS: These cases highlight the existence of a new form of linear IgA bullous dermatosis presenting as a morbilliform drug eruption. Both patients were following extensive medication regimens, including use of multiple antibiotics. The diagnosis of linear IgA bullous dermatosis allowed us to target vancomycin as the likely allergen and begin treatment. In light of these findings, direct immunofluorescence may be a useful diagnostic adjunct in determining the cause of drug eruptions.

We report the case of a 69-year-old Japanese woman with multiple blistering lesions covering almost her whole body. Linear IgA and C3 depositions were seen at the basement membrane zone on direct immunofluorescence (IF). Linear IgA bullous dermatosis (LABD) is one of the autoimmune diseases resulting in subepidermal blisters.

It is clinically similar to bullous pemphigoid and IF is required to distinguish the two diseases. In this case, the blistering lesions appeared after vancomycin treatment. This drug was strongly suspected as a cause of LABD in light of the clinical course of the patient even though a drug-lymphocyte stimulating test was negative.

Among the various implicated causative drugs, vancomycin is the most commonly associated with LABD.

Background: Vancomycin-induced linear immunoglobulin A (IgA) disease, an autoimmune, blistering disease in response to vancomycin administration, is characterized by a subepidermal, vesiculobullous eruption and linear IgA deposition along the basement membrane zone on direct immunofluorescence.

Case report: We report the case of an 81-year-old man treated with vancomycin who developed diffuse erythema multiforme and tense bullae involving the palmoplantar surfaces. Discontinuation of vancomycin therapy resulted in complete resolution of this patient's cutaneous eruption.

A 69-year-old
woman presented with pneumonia and subacute bacterial endocarditis.
Nine days after intravenous vancomycin and ciprofloxacin were commenced,
the patient developed a bullous mucocutaneous eruption.

A review of the features of drug-induced LABD and the drugs that have
been implicated are presented.

Linear IgA bullous dermatosis induced by atorvastatin

Cathrin König, etal.

J Am Acad Dermatol 2001;44:689-92 Abstract quote

Linear IgA bullous dermatosis (LABD) is an autoimmune blistering skin
disease characterized by circulating IgA antibodies binding the basement
membrane zone. In most cases the origin is not clear, but in a minority
of cases LABD is drug induced.

We describe a patient in whom linear IgA disease developed shortly
after beginning therapy with atorvastatin. In Western blotting analysis
we detected IgA and IgG class antibodies targeting a 97-kd protein.

To our knowledge this is the first reported case of atorvastatin-induced
LABD.

Linear IgA bullous dermatosis in one of two piroxicam-induced
eruptions: A distinct direct immunofluorescence trend revealed by the
literature

Background: The report focuses first on two patients with piroxicam-induced
bullous eruption, one whose disease was diagnosed as linear IgA bullous
dermatosis (LABD) and the other with no disease-specific immunologic
findings using immunofluorescence methods. A review of the literature
points to a distinctive direct immunofluorescence feature of drug-induced
LABD cases.

Objective: Our purposes were to focus on divergent piroxicam reactions
and to compare immunofluorescence findings in our and other reported
drug-induced LABD cases to randomly occurring LABD cases.

Methods: Direct and indirect immunofluorescence methods were used to
study biopsy and serum samples from both cases and biopsy specimens
of 40 other LABD cases.

Results: Tense blisters developed in two patients medicated with piroxicam.
Immunofluorescence studies demonstrated deposits of IgA at the basement
membrane zone (BMZ) in case 1 and only non-disease-specific fibrin deposits
at the BMZ in case 2. Within 1 month of discontinuation of piroxicam,
all lesions were gone in both patients.

Conclusion: In LABD cases proven by direct immunofluorescence, (1)
the index of suspicion of drug induction should be higher in cases with
only IgA and no IgG in the BMZ; (2) possibly up to two thirds of all
LABD cases may be drug induced; and (3) the negative immunofluorescence
findings in case 2 and other cases reported in the literature suggest
that LABD is one of several host responses in drug-induced blistering
diseases.

Linear IgA dermatosis and Hodgkin's lymphoma--report of a case in an
African and review of the literature.

Jacyk WK, Nagel GJ, van der Hoven AE.

Department of Dermatology, Kalafong Hospital, Pretoria, Republic
of South Africa

J Dermatol 1990 Oct;17(10):633-7 Abstract quote

Linear IgA dermatosis
and Hodgkin's lymphoma were diagnosed at the same time in a 47-year-old
Black South African man. Skin changes of linear IgA dermatosis responded
to a combined treatment with dapsone and MOPP regimen. Prior administration
of dapsone alone did not result in improvement of the skin condition.

Four documented cases of linear IgA dermatosis and lymphoma outside
the gastrointestinal tract, three of them of Hodgkin's type, have been
reported in the literature. Review of the literature suggests that different
forms of lymphoma seem to be associated with linear IgA dermatosis and
dermatitis herpetiformis.

The present patient represents the first immunologically verified
case of linear IgA dermatosis in an adult Black African.

BACKGROUND:
Although many patients with linear IgA dermatosis (LAD) are young and
have persistent disease, little is known about the interactions between
LAD and pregnancy.

OBJECTIVE: Our purpose was to study the effects of LAD on pregnancy,
and vice versa.

METHODS: Our study included 12 patients with LAD who underwent a total
of 19 pregnancies.

RESULTS: In all patients the disease improved during pregnancy, enabling
therapy to be reduced or stopped. Dapsone was taken by patients during
11 pregnancies, and no adverse effects were seen. No patients had problems
in labor. Most patients had a relapse approximately 3 months post partum,
even if they had previously been in remission. In two patients, disease
started within 3 months of delivery. Fetal outcome was unaffected in
all but one fetus, who had a single transient blister.

CONCLUSION: We found no contraindication to pregnancy in patients with
LAD. We recommend that therapy be reduced or stopped whenever possible
during pregnancy and that patients be counseled about the possibility
of a relapse post partum.

Linear IgA
disease is an acquired bullous disease of the skin characterized by
linear IgA deposits along the dermal-epidermal junction. Inflammatory
bowel diseases have been rarely reported in association with linear
IgA disease.

We have recently observed a patient suffering from ulcerative colitis
who developed a cutaneous bullous eruption that was diagnosed as a linear
IgA disease. Foreign antigens penetrating the inflamed bowel mucosa
might give rise to the production of autoantibodies cross-reacting with
the cutaneous antigens involved in the pathogenesis of linear IgA disease.

Collagen XVII, or BP180, is a collagenous transmembrane protein and a structural component of the dermoepidermal anchoring complex. Molecular studies reveal that it has a globular cytosolic amino-terminal domain and flexible-rod extracellular carboxyterminal domain. The extracellular portion of collagen XVII is constitutively shed from the cell surface by ADAMs (proteinases that contain adhesive and metalloprotease domains). Cell biological analyses suggest that collagen XVII functions as a cell-matrix adhesion molecule through stabilization of the hemidesmosome complex.

This concept is supported by investigations into human diseases of the dermoepidermal junction, in which collagen XVII is either genetically defective or absent (as in some forms of nonlethal junctional epidermolysis bullosa). Autoantibodies against collagen XVII (BP180) are seen in bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA disease, lichen planus pemphigoides and pemphigoid nodularis. In vivo and in vitro studies provide evidence for a pathogenic role of these autoantibodies, and suggest that the serum level and epitope specificity of these antibodies influences disease severity and phenotype.

This review summarizes the structural and biological features of collagen XVII and its role in diseases of the basement membrane zone.

Mapping of epitopes on the BP180 ectodomain targeted by IgA and IgG
autoantibodies in patients with the lamina lucida-type of linear IgA
disease.

Linear IgA disease (LAD) is an autoimmune subepidermal blistering skin
disease characterized by the linear deposition of IgA at the dermoepidermal
junction.

Serum from patients with LAD most commonly contains autoantibodies
that are directed against the hemidesmosomal transmembrane glycoprotein
BP180 (type XVII collagen). Various antigenic sites on the extracellular
domain of this anchoring filament protein have been shown to be targeted
by autoantibodies in different autoimmune bullous skin diseases, including
bullous pemphigoid and cicatricial pemphigoid (CP).

However, little is known about epitopes on BP180 recognized by autoantibodies
in LAD. In this study, we used three recombinant GST fusion proteins,
together roughly covering the entire BP180 ectodomain, to characterize
the autoimmune response in serum from patients with LAD. Interestingly,
we found both IgA and IgG reactivity to all three portions of the BP180
ectodomain. The strongest reactivity was observed with the C-terminal
portion of BP180. This is also the major region recognized by autoantibodies
in patients with CP.

This finding correlates with the observation that there may be significant
overlap of the clinical and immunopathological findings in LAD and CP.

Autoimmune responses in patients with linear IgA bullous dermatosis:
both autoantibodies and T lymphocytes recognize the NC16A domain of
the BP180 molecule.

Various antigens have been identified as targets of IgA autoantibodies
including BP180, a type II glycoprotein that spans the BMZ and lamina
lucida. Previously, we have identified a subset of LABD patients whose
sera contained IgA antibodies against the 16th noncollagenous (NC16A)
domain of BP180. NC16A was previously shown to harbor epitopes that
are recognized by both autoantibodies and T cells from patients with
bullous pemphigoid and herpes gestationis and is thought to be associated
with the development of these immunobullous diseases.

The aim of this study was to determine whether T lymphocytes from LABD
patients with anti-NC16A IgA autoantibodies respond to epitopes in the
same region of the BP180 protein. Indeed, of the four LABD patients
in our study, all had T cells that specifically proliferated in response
to NC16A. Moreover, two subfragments of NC16A were identified as the
predominant targets of LABD T cells. Further analysis of T cell lines
and clones derived from these patients revealed that these cells express
a CD4 memory T cell phenotype and secrete a Th1/Th2 mixed-cytokine profile,
characteristics similar to those of T cells in bullous pemphigoid patients.

Our data suggest that the BP180 protein, typically the NC16A region,
is the common target of both cellular and humoral immune responses in
some LABD patients. This information helps to further elucidate the
autoimmune mechanisms in this disease.

A multi-centre
study is described in which thirty-five adult patients with papillary
IgA dermatitis herpetiformis (DH) were compared with forty-two patients
with linear IgA deposits, of whom thirty-four had homogeneous-linear
(HL) and eight had granular-linear (GL) IgA deposits.

The three groups were similar with regard to age of onset, presence
of circulating immune complexes and auto-antibodies, incidence of spontaneous
remission, histology of lesional skin and response to dapsone. There
was a female predominance in the HL group in contrast to the male predominance
in the other two. It was not possible to diagnose the HL group clinically.
Some patients had a rash typical of DH whilst others resembled pemphigoid.
In the majority, however, no specific diagnosis could be made with confidence.
The GL group clinically resembled the DH group. The incidence of positive
potassium iodide patch tests was greater in the DH group than in the
other two. An associated enteropathy was found in 24% of patients in
the HL group, 30% of patients in the GL group and 85% of patients in
the DH group. Fifty-six percent of HL patients had HLA-B8 compared with
50% in the GL group and 88% in the DH group.

Patients with linear IgA deposits may not be a uniform group, but until
they can be divided into specific subgroups (e.g. by ultrastructural
localization of the deposit or by response to a gluten-free diet) we
propose that the term adult linear IgA diseases should be used to distinguish
these patients from those with papillary IgA deposits.

Linear
IgA disease of adults, chronic bullous disease of childhood, and the
rare childhood cicatricial pemphigoid currently are regarded as separate
clinical entities despite their many shared features. All are sulfone-responsive
subepidermal bullous diseases associated with linear IgA deposition
at the basement membrane zone.

In this paper we present a long-term study of 25 cases of adult linear
IgA disease, 25 cases of chronic bullous disease of childhood, and four
cases of childhood cicatricial pemphigoid, which has revealed further
similarities among all three groups. The morphology and distribution
of the cutaneous and mucosal lesions were similar; mucosal involvement
was present in 80% of patients with adult linear IgA disease, 64% of
those with chronic bullous disease of childhood, and 100% of those with
childhood cicatricial pemphigoid, and ocular scarring affected patients
in all groups.

Remission occurred in 64% of those with chronic bullous disease of
childhood (the disease was active in 12% after puberty), 48% of those
with adult linear IgA disease, and in no cases of childhood cicatricial
pemphigoid. HLA B8 and circulating IgA anti-basement membrane zone antibody
were more common in chronic bullous disease of childhood than adult
linear IgA disease.

There were no absolute differences among the three groups, and we
suggest that adult linear IgA disease, chronic bullous disease of childhood,
and childhood cicatricial pemphigoid are the same disease, with childhood
cicatricial pemphigoid being a more severe form of chronic bullous disease
of childhood.

VARIANTS

CHILDHOOD

Benign chronic bullous dermatosis of childhood: a review.

Sweren RJ, Burnett JW.

Cutis 1982 Apr;29(4):350-2, 356-7 Abstract quote

Chronic bullous
dermatosis of childhood is one of the nonhereditary blistering diseases
of children. Clinically, it is characterized by predominantly monomorphous,
large tense bullae, which often form a "rosette pattern" or
"jewel-like" clustering and have a predilection for the lower
trunk, pelvic region, and lower extremities.

Histologically, a subepidermal blister is seen, which is indistinguishable
from either bullous pemphigoid or dermatitis herpetiformis. Although
usually responsive to sulfone therapy, some cases require the combination
of sulfones and systemic corticosteroids or corticosteroids alone to
control the disease.

Recent advances in immunologic techniques reveal: 1. a linear band
of IgA at the dermal-epidermal junction on direct immunofluorescence
that has been reported both in the lamina lucida and below the basal
lamina on immunoelectron microscopy; 2. IgA antibasement membrane antibodies
on indirect immunofluorescence; 3. normal jejunal biopsies; and 4. a
high association with HLA-B8. It remains unclear whether chronic bullous
dermatosis of childhood represents a separate disease entity or is merely
a variant of dermatitis herpetiformis.

Chronic bullous dermatosis of childhood also differs from linear IgA
dermatosis of the adult in that the latter is not associated with HLA-B8,
and thus should not be confused with this disease by similar nomenclature.

Of twenty-seven
cases of subepidermal blistering disease of children twelve corresponded
clinically, histologically and immunologically to dermatitis herpetiforms
of adults, six to bullous pemphigoid, and eight to chronic bullous disease
of childhood (CBDC), i.e. IgA linear dermatosis.

This latter disease seems to be a distinct entity, different from both
dermatitis herpetiformis and bullous pemphigoid, and is characterized
immunopathologically by linear IgA deposits at the basement membrane
zone. These cases usually do not show intestinal involvement and respond
well to combined treatment with sulphones and corticosteroids, whereas
sulphones or sulphapyridine alone are, even in very high doses, not
sufficient for full control of the disease.

CBDC or IgA linear dermatosis of childhood may be regarded as a counterpart
of IgA linear dermatosis of adults.

Department of Dermatology, University Medical Center Benjamin
Franklin, The Free University of Berlin, Germany.

J Eur Acad Dermatol Venereol 2000 Mar;15(2):167-70 Abstract quote

Linear
IgA dermatosis presented with erythema annulare centrifugum lesions
in three elderly women. Search for underlying malignancy revealed low-grade
B-cell lymphoma in one case. In addition to subepidermal blistering,
histology showed a typical mixed infiltrate of granulocytes and eosinophils
and, occasionally, papillar microabsesses in one case. In the two other
subjects, characteristic subepidermal lining with granulocytes was observed.
Immunofluorescence studies confirmed the diagnosis, while autoantibodies
characteristic for dermatitis herpetiformis were absent.

To our knowledge this is the second report of adult linear IgA dermatosis
in association with erythema annulare centrifugum lesions. Our observations
concord with several other reports of figurate erythema associated with
autoimmune blistering disease and other immune disorders.

Common antibody-related immunological mechanisms indicate that the
two distinct clinical pictures are probably stages of the same pathogenic
entity.

LARYNX/PHARYNX

Initial presentation and fatal complications of linear IgA bullous dermatosis in the larynx and pharynx.

Two cases of linear IgA bullous dermatosis initially presenting as ulcerative lesions in the larynx and pharynx are reported. It was difficult to diagnose and treat the lesions, but they were finally diagnosed from the histopathological findings of accompanying skin lesion specimens.

One of the patients required a tracheostomy due to increased airway stenosis by a laryngeal lesion. Despite general corticosteroid administration this could not be completely resolved, although partial opening of the glottis was observed, and the patient died of accidental tracheostomy tube complications during home care.

Although there are no reports of this disease in the otolaryngological field, these rare diseases involving the skin and entire body should be considered in the differential diagnosis of laryngeal and pharyngeal ulcerative lesions, including airway stenosis.

Furthermore, simple and safe procedures for relieving airway stenosis should be selected for rare and difficult-to-diagnose airway disease, prior to the final diagnosis.

ORAL

Oral manifestations of linear IgA disease.

Chan LS, Regezi JA, Cooper KD.

Department of Dermatology, University of Michigan Medical Center,
Ann Arbor

J Am Acad Dermatol 1990 Feb;22(2 Pt 2):362-5 Abstract quote

A case
of linear IgA disease with prominent oral lesions is presented. Oral
manifestations in linear IgA disease have been reported as minor clinical
presentations.

In our patient the oral manifestations predominated and were the only
clinical manifestations for 5 years before the skin lesions appeared.
Linear IgA disease should be included in the differential diagnosis
of bullous dermatoses with oral lesions.

A case of desquamative gingivitis caused by adult linear IgA disease
is presented. Management initially proved to be difficult, however,
the introduction of sulfapyridine caused rapid resolution of the gingival
problem.

This is one of the first reports of desquamative gingivitis caused
by linear IgA disease successfully treated with sulfapyridine.

HISTOLOGICAL TYPES

CHARACTERIZATION

SKIN

Subepidermal or subepithelial bullous dermatosis
Collections of neutrophils, usually around the base of rete ridges

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER

CHARACTERIZATION

Direct immunofluorescence of skin (DIF) and salt split
skin

Biopsy should be taken from perilesional skin or mucosa
with normal and edge of lesional areas

If DIF is consistent with LABD, do salt split skin test to differentiate
from epidermolysis bullosa form of LABD

Histopathologically, 5 of 7 patients with LAD were similar to the DH
group, but 7 of 10 patients with LAGD were similar to the BP group.
Half the patients with LAD and LAGD had oral lesions, and most of them
had excellent responses to dapsone and Tripterygium Wilfordii, but the
patients with CBDC did not respond to these treatments.

In the patients with LAD and LAGD, the positivity rates of IgA anti-BMZ
antibodies examined by indirect immunofluorescence (IIF) on intact skin
and NaCl split skin were 41% and 64%, respectively. The heterogeneity
of the histopathologic pictures of LAD and LAGD, the incidence of DH,
and the value of using NaCl split skin for IIF are discussed.

Linear IgA disease with clinical and immunopathological features of
epidermolysis bullosa acquisita.

Mutasim DF, Cummings MP.

Department of Dermatology, University of Cincinnati, Ohio 45267-0523,
USA.

Pediatr Dermatol 1997 Jul-Aug;14(4):303-6 Abstract quote

A 10-year-old boy had a 3-month history of urticarial plaques and vesicles.
Histologic and immunofluorescence testing confirmed the diagnosis of
linear IgA disease.

Immunoelectron microscopy revealed IgA deposits in the sublamina densa
area similar to those seen in epidermolysis bullosa acquisita. Milia
developed after resolution of the lesions, similar to lesions of epidermolysis
bullosa acquisita.

PROGNOSIS AND TREATMENT

CHARACTERIZATION

Prognostic Factors

Epidermolysis bullosa acquisita form may be difficult to
treat

TREATMENT

Idiopathic form may be more difficult to treat than drug
induced form

Usually use dapsone and prednisone

ANTIBIOTIC-FLUCLOXACILLIN

Treatment of linear IgA bullous dermatosis of childhood with flucloxacillin.

BACKGROUND: Linear IgA bullous dermatosis of childhood is a rare autoimmune bullous disease that mainly affects preschool-aged children. Dapsone is considered the first-line therapy with prompt response from most patients. However, it may be contraindicated in certain conditions such as glucose-6-phosphate dehydrogenase deficiency.

OBJECTIVE: We sought to assess the efficacy of flucloxacillin in the treatment of linear IgA bullous dermatosis.

METHODS: This is an observational study in which all confirmed cases of linear IgA bullous dermatosis (by both histological and immunofluorescence studies) will be treated with flucloxacillin. Flucloxacillin will be continued according to the response or otherwise will be discontinued after 8 weeks in the case of resistance.

RESULTS: We describe 7 patients with linear IgA bullous dermatosis of childhood treated with flucloxacillin. In 4 cases, it induced complete remission within 3 to 4 months of starting therapy with no relapses. In the other 3 cases, it successfully controlled the disease but with prompt relapse on discontinuation of the treatment.

LIMITATIONS: This is a case series study with a small number of patients.

CONCLUSION: Flucloxacillin may be considered among the first alternative therapies for linear IgA bullous dermatosis of childhood. Further evaluation of the efficacy and safety of the long-term use is required.

GLUTEN FREE DIET

Experience with a gluten free diet in the treatment of linear IgA disease.

Leonard JN, Griffiths CE, Powles AV, Haffenden GP, Fry L.

Acta Derm Venereol 1987;67(2):145-8 Abstract quote

A study was undertaken to determine whether the skin eruption of linear
IgA disease (LAD) was gluten dependent.

Six patients with LAD were treated with a gluten free diet (GFD) for
an average period of 33 months (range 19-48). Although one patient with
LAD had an enteropathy which was clearly gluten sensitive, there was
no convincing evidence that the rash of any of the patients responded
to a GFD. Four of the six patients showed no significant alteration
in their drug requirements. The remaining 2 patients showed a fall in
minimum drug requirement but there was no increase after gluten challenge
indicating that they were entering spontaneous remission.

This contrasts to the situation in dermatitis herpetiformis, where
both the rash and the enteropathy are gluten dependent. These data add
further to the evidence that LAD and dermatitis herpetiformis are separate
entities.

Dermatitis
herpetiformis is associated with a gluten-sensitive enteropathy in >85%
of cases. Both the skin lesions and the enteropathy respond to gluten
restriction. Linear IgA bullous dermatosis has a much lower prevalence
of histological small bowel abnormalities, and lesions are not known
to respond to gluten restriction.

We report a patient with linear IgA bullous dermatosis and gluten-sensitive
enteropathy. This report addresses the issue of whether linear IgA bullous
dermatosis can be associated with gluten-sensitive enteropathy. We evaluated
the response to gluten restriction and normal diet by following the
status of the patient's jejunal biopsies and skin lesions. The patient
responded to gluten restriction, as shown by resolution of jejunal abnormalities
and skin lesions and subsequently by recurrence of jejunal abnormalities
and skin lesions with reinstitution of a gluten-containing diet.

This report demonstrates that linear IgA bullous dermatosis can respond
to gluten restriction if an underlying gluten-sensitive enteropathy
is present.

PURPOSE: To report
on a diagnostic dilemma and treatment challenge in a patient with chronic
cicatrizing conjunctivitis without involvement of skin and other mucous
membranes persisting for 6 years and not responding to topical and systemic
steroids.

DESIGN: Interventional case report.

METHODS: We performed direct immunofluorescence of the conjunctiva
with fluorescein-conjugated rabbit antihuman antibodies against immunoglobulin
A, G, and M, complement 3 component, and fibrinogen. To investigate
the presence of circulating antibodies in patient's serum, indirect
immunofluorescence using normal human conjunctiva, normal human skin,
and monkey esophagus as substrate was done. In addition, we did immunoblot
analysis using normal human epidermis as substrate to determine the
molecular weight of an antigen. The patient was treated with intravenous
immunoglobulin (IVIg). The correlation between the titer of circulating
antibodies and the activity of conjunctival inflammation at various
intervals during the course of IVIg therapy was demonstrated by immunoblot
assay with serial dilutions of the patient's serum. The highest dilution
at which the binding was visible was considered the titer.

RESULTS: Direct immunofluorescence of the conjunctiva and indirect
immunofluorescence with both salt split skin and conjunctiva as substrate
disclosed linear deposition of immunoglobulin A (IgA) at the epithelial
basement membrane. Immunoblot analysis demonstrated the presence of
IgA circulating antibodies in patient's serum directed against a 97kDa
protein in human epidermis. A continuous decrease in the titer of these
antibodies correlating to improvement of clinical symptoms was observed
during IVIg therapy.

CONCLUSIONS: Use of a nonconventional diagnostic tool (immunoblot analysis),
in addition to conventional immunohistologic studies, might be helpful
in establishing the diagnosis of patients with chronic cicatrizing conjunctivitis.
On the basis of results of these laboratory tests and clinical presentation,
we believe that this patient has linear IgA bullous disease limited
to the eye. IVIg therapy decreased the titer of circulating antibodies
and induced a remission in this patient.